Question about BHS/POTS/Ehlers-Danlos

I am new to this site and have some questions. I have fibromyalgia + was sent to a rheume who specialized in fibro. He diagnosed me also with Benign Hypermobility Syndrome (BHS). He told me to look it up for questions at my next visit. What I found was that it is a type III Ehlers-Danlos, but when I asked him about it, he said I did not have Ehlers-Danlos but I do have BHS. I did not understand. I also have VonWillebrand's (I'm a bleeder) have IBS, migraines, sleep disorder, Chronic Fatigue and more recently have had what my heart doctor and PCP think is POTS. My problem is that I have near syncope (typically) but have have 2 syncopal events within the past month. My BP had been low. They had me eating salt and on multiple trials of Rx meds to bring the BP up (plus fluids). The episodes still would occasionally still occur. Certain meds made the BP too high. Subsequently my heart doc took me off the med and told me that I now have developed hypertension (limit salt). He has not started me on HBP med yet until I see another heart doc who specializes in dysautonia (which may be months from now). Heat, activities where I am stooped or kneeling or bent down (like pulling weeds), and sometimes I don't know what other trigger are that make me faint. My BP drops and the heart rate goes anywhere from the 120s to 150s after I check them after I've rested from the episode. My questions are: does my having been diagnosed with BHS put me in Ehlers-Danlos? If it does, I will want to add that to my health history (as none of my docs really know anything about BHS). Is POTS common in Ehlers-Danlos? I am so tired all day. These 'spells' as I refer to them to my grandson who was with me when I had one (when he + I were on our bikes/alone and he was 4 but he just turned 7) make me very reluctant to be entrusted to babysitting my grand-daughter who just turned 3 and is always on the move. I did have 2 tilt table tests and a month of the heart monitor. The heart monitor test wasn't even completed when the heart doc called saying I had some kind of tachycardia (and he Rx'd med--but I would still occassionally have the near syncope, so he took increased the dose. Same thing. He changed the med. Same thing. Then no meds but still spells and he decided it was from dysautonia and now has determined it to be POTS). Since I am on no Rx for the HTN and it seems that activity makes the BP go higher (and I am not a very active person), I live guarded with what I do until this heart specialist sees me. There is so much I do not understand, but anyone who might be able to answer my questions, I want to thank you in advance.

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I am sorry to hear you are having so many problems getting your tachycardia and blood pressure straightened out. Have you seen a geneticist familiar with EDS yet? Perhaps someone in your area can refer you to one. Brad Tinkle in Cincinnati, OH, is excellent. (Really--one of the best for EDS.) http://www.cincinnatichildrens.org/svc/find-professional/t/brad-tinkle.htm

POTS definitely is common in EDS.

BHS is on the spectrum of EDS hypermobility. The fact that you have IBS, migraines, chronic fatigue, sleep disorder and POTS makes me think that an EDS expert would say you have Hypermobility EDS and not BHS, but that's for an expert who has you in front of him to say, not me.

Make your doc write down what specifically it is that he thinks makes you BHS and not EDS hypermobility. That should help you clarify the issue when seeing other specialists.

Read this article and make your doc do the same: Tinkle BT, Bird HA, Grahame R, Lavallee M, Levy HP, Sillence D. The lack of clinical distinction between the hypermobility type of Ehlers-Danlos syndrome and the joint hypermobility syndrome (a.k.a. hypermobility syndrome). Am J Med Genet A. 2009;149A:2368–70. (Do a search on www.pubmed.com.)

I highly recommend trying to see Dr. Tinkle. Also, if you can come to the EDNF conference in July, you'll probably learn a lot. Go to www.ednf.org for more info.

In the meantime, know that your instincts regarding what brings on your syncopal episodes are correct. Staying in one position for a long time, bearing down, changing heights quickly (e.g., kneeling to standing), hot weather, dehydration, and a number of other things can precipitate syncopal episodes. Avoid known triggers during this period while the docs are trying to figure out medications, etc. Certain medications (including pain medications) can also cause issues. The issue in POTS is that the heart rate will skyrocket in order to keep the blood pressure sufficiently high. When they test your blood pressure in the office, make sure they wait a sufficient amount of time after you have come into the office and sat down before checking your blood pressure. Accurate measurements are key to this assessment. It is fairly unusual for an individual with POTS to have high blood pressure EXCEPT during compensatory/rebound spikes.

POTS can be frankly dangerous. While fainting, I have hit my head on concrete, wood floors, and walls. I have become disoriented while driving and have had to pull over in less than favorable spots. Because of this, I urge you to push this issue with your doctors, or better yet, get to a specialist who can do an appropriate job of balancing the risks of POTS with the risks of high blood pressure. If Dr. Tinkle cannot get you in soon, perhaps he can refer you to someone who can address the EDS/BHS-related POTS in a reasonable amount of time. At the very least, the person he refers you to will surely be qualified to figure this situation out.

Best of luck to you.

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I want to totally agree with all that Red Balloon has said and encourage you to keep searching until you get the right doctor. Your life will be simpler with proper health-care management. Just knowing the facts has helped me identify what I need to do and how to address the hereditary issues involved with my dx.

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Let me respond to a different part of your post.

I'm a mommy of a 5 y.o. and have been dealing with this since he was 2 1/2. (EDS II and POTS)I had issues with driving and his care. Eventually I had to hire a "mother's helper" which is still a sitter but works with a parent (or grandparent) in the home. I know the idea of YOU watching your grand-daughter was likely for the parents to save some money. But... if you're anxious about driving or keeping up with her, the anxiety can be a trigger, too. It's summer and you could probably find a mother's helper pretty cheap (can be a junior high/high school student) who can be in the house and more of the physical stuff. But then you can still make lunch (then lie down), help with the potty (then lie down) and pull out her favorite coloring books (and then lie down again).

It's just a suggestion. We didn't have to put my son into daycare and I still got some good bonding time. I just had someone else chase him around. My "helper" did some light cleaning, too!

So sorry you're dealing with this. Hope you get some comfort soon. By the way, I didn't tolerate POTS meds. Lots of fluids, gatorade and salting my food helps. Don't over-exert yourself and stay cool. I put a cold pack on my feet and my head on hot days.

:-)

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I failed to mention that, though it took me a long time to figure out how to effectively modulate my POTS symptoms, I now have, and am much more functional. I didn't want to make you think that this was some sort of death sentence. Keep working to get the situation under control. It can happen:) Some medicines, some lifestyle modification, some precautionary measures all combine to make a big difference.

A lot of my improvement has to do with getting off of certain pain medicines, learning how to control my diarrhea, learning how to prepare for POTS dangerous situations (e.g., warm weather, exercise, standing around, not enough sleep, anesthesia), and learning to deal aggressively at the earliest signs of dehydration. And doing the "lie down" thing Mommy dancer mentions!

One measure I highly recommend to everyone with POTS is asking for IV hydration--with saline plus glucose--prior to surgeries and other procedures. If I can't eat or drink prior to a procedure, there is no way that my POTS won't be causing a problem during the procedure. I used to be gun shy about asking for IV hydration prior to procedures until I nearly died because my bloodpressure and heartrate tanked during the administration of anesthesia at the start of a surgery. When the surgery was attempted again a month later, the surgeon actually insisted that I be given 2 L of IV saline with glucose prior to being wheeled into the OR. I didn't have to say anything.

I imagine I had trouble in that particular surgery because the anesthesia was administered a bit too quickly, but I had begged them prior to that surgery to be careful with the anesthesia and to administer it slowly, so know that such requests only go so far, even with doctors who have excellent reps.

Even in procedures where you won't be out completely, IV hydration is helpful because it brings up your blood pressure allowing them to administer more pain medicine. Think of colonoscopies, eye surgeries, etc.

Again, good luck!

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Thank you for the suggestions and resources, red balloon. I just e-mailed Dr. Tinkle to inquire if he could suggest a doctor to see me for an evaluation. Not being a pediatric patient, I just assumed he doesn't see adults in his practice but perhaps he might know of someone. I have had the BHS listed on my health history that has been given to every physician I see and none have ever questioned it. I even asked my ortho (who was the one who referred me to the rheume who specialized in fibro) about his diagnosis of BHS and my orthoped was not familiar with it (we live in a rural area). Until my sister sent me a link to a post about POTS when whe researched it (and she did not know I had been diagnosed with BHS), I accepted what the rheume said, but now am skeptical that perhaps he was not so familiar with EDS Hypermobility.
I would like to go to the conference but travel is very painful for me since the accident (my glutteal muscles and tendons were torn off the hip bone). I stay in constant pain (that my ortho attributes to fibro and chronic myofascial pain and osteoarthritis) from that injury but prefer not to take Rx'd pain meds due to their effects on me. I have multiple drug allergies and cannot take the non-steroidal antiinflammatories. I have long thought there must be some connection with all of these afflictions, as if my body nervous system or something were wired wrong, or that it is something immunologic. I see an infectious disease doctor at Kettering for osteomyelitis and my T-helper cells are low (but he doesn't know why). All the battery of lab tests and ultrasounds and echocardiograms are normal (except for a little thickening on the heart valves and a small pericardial effusion that nobody knows why I have).
I do find that my syncopal events seem worse in the summer and usually find myself indoors in the cool AC. I just don't seem to be able to squat down to pull a few small weeds without it starting. But then even doing the postural changes in housekeeping can cause the same symptoms--its just that I've learned to recognize the feeling and get down right away. I wonder if the constant pain is having an effect in making me now have HTN (but since I had always run a BP a little on the low/low normal side until I'd been on trials of all those meds and all that salt, if that hasn't set me up for this HTN). I used to ride my bike 10 miles/day (early before the heat of the day) or I would workout on the treadmil 30 minutes/day but until the POTS/HTN treatment is established and stabilized, I don't do that (the activity causes my BP to rise and my HR to rise too high). And I will sweat buckets standing in a yard when nobody else has a bead of sweat on them.

My mother has had syncopal episodes that were never really diagnosed (until they happened upon her having pulmonary emboli) but even years later, when I describe my 'spells' she tells me she has the same thing (so again when reading this forum, I wondered about genetics). I have always told her that she might have what I have.

I have also read that many insurance plans (and ours is frugal) do not cover genetic testing, and that genetic testing for this has about a 50% accuracy? There are no genetecists (or specialists) around my neck of the woods but I am hoping the good doctor you suggested might be helpful.

Thank you again.

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Leslie62,
Thank you for your support. Just how is your treatment changed by your physicians from before and after being diagnosed with EDS III? Don't they still just more or less treat symptoms, or is there something different they watch or check or treat?

Thank you for your help.

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mommydancer,
I don't get to see my grand-daughter but a few times a year and it was only this year that her mother has let her come over. I've been trying to bond with her (and with one going through the terrible twos it is challenging). A mommy helper isn't in our budget but I agree it is a great option for those who can take advantage of that. She is constantly on the go, very independent (the 'baby' of the family who appears to rule the roost and throws tantrums until she has her way). I never had those issues with my g-son--exact opposites. I ignore (do not positively reinforce) her tantrums and eventually, she sees things are not going her way. After she settles down from the fit, then she lets me help her and we're best friends. But it does create anxiety--plus I cannot run fast enough to beat her to the door and she can reach/open them. She doesn't listen and does not mind at all. I'm not saying she's a bad girl (please don't get me wrong), it's just that she's a toddler who wants to have her independence. The hardest part is she loves the outside/swinging and there is no shade and I have to push her and stand and that gets the heart racing and the lightheadedness starts and she won't take 'wait' or 'not now' for an answer. When she was here for her b-day, she didn't want to go home--wanted to stay all night but her mother wanted her home. She ran inside the house as if she could hide or position herself soas not to be taken away. It took us over an hour to get her home (and my husband reinforced her tantrum/behavior by getting her a happy meal on the way home). As much as I want our alone time to bond, and as much as I would love to see her more, I know that for her sake and for mine, I have to consider my health first. Thus, I have not made myself readily available for visits unless my husband or grandson can be here at the time of her visits.

Thank you again for your support.

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rubytuesday1-

Dr. Tinkle absolutely sees adults. I just saw him a few weeks ago. I couldn't get an appointment offsite but I DID get to see him at Cincinnati Children's Hospital. Also, my POTS doctor is one of the world's best: Dr. Blair Grubb in Toledo. (I live in Michigan) They're both speaking at the EDS Conference. It takes a while to get in (with both docs) but it's worth the wait. Get on their patient lists and in the meantime, trial and error to see what works for you.

I completely understand regarding your grand daughter. They're lovely but it's a tough age.

Gentle hugs.

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red balloon,

That is strange you should mention about anesthesia. I had one major surgery and found myself intubated but unable to respond in any manner (could not open my eyes or flinch or moan an utterance) when the nurses repeatedly called my name and even were doing sternal rubs/pinches in attempts to get a pain reaction. Finally, I abruptly sat up and coughed out the airway (balloon still inflated) when the nurses were on the phone with my doctor that they could not get me awakened. I could hear and feel everything that was going on but couldn't move at all. Other times they have had difficulty with my arrousal and my oxygen level coming up quickly enough. Since that first event I mentioned, I forewarn every anesthetist before my surgery. I'm always given IV fluids before the procedures but I never lookes to see what it was--just assumed it was saline.

I don't take my pain meds because I don't like being more drowsy when I'm tired all the time, the rebound headache I wake up with the next day and then suffering days of constipation for taking 1 pill, then subsequently an entire 'bathroom day' when the contipation resolves. Since all of this I now have thrombosed hemorrhoids and a fistula (despite taking the miralax as my doctor suggested, and before that, metamucil, colace and mineral oil that a surgeon suggested, and sometimes I have to take the miralax and mineral oil). I live with the pain every minute of every day since the glutteal muscles/tendon were torn off the hip bone when I was injured. I even asked the heart doctor if the pain could trigger some of these events (I mean a body can only take so much) but he did not think so. I am getting more aware of things that cause the symptoms and get down as quickly as I can before it progresses (and it can progress without enough time to prevent it). But it is limiting as often--especially when trying to do the housekeeping--I have to stop and sit so many times before I can go and continue--which is also stressful when you want to get the job done. I already have to pace myself because of the fibro/chronic fatigue--but then maybe it is POTS, not CFS.

Thank you again.

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Dr. Tinkle sees adults.

DNA testing (the sort that you're probably referring to when you say that insurance companies don't pay) is not required for a diagnosis of EDS. There isn't even such a test for Hypermobility EDS.

I am so sorry to hear about your osteomyelitis, T-helper-cell issue, pericardial effusion, and recent accident. It sounds like you have your hands quite full. Honestly, more that just the regular EDS-causes of POTS could be going on in your case. Please be sure Dr. Tinkle knows about these things prior to his scheduling you for an appointment at his clinic or referring you to someone else. Perhaps things didn't start that way, but am suspicious of the role that pericardial effusion would play now in your POTS. Depends on the degree, I suppose.

There are lots of options for pain management that do NOT involve sedating medications or NSAIDS. Look under the pain management category. When looking for a good pain doctor, get a referral from someone with EDS or from Dr. Tinkle. Every surgeon in the world seems to have a recommended list of pain docs that is 75-100% bad eggs, at least as far as EDSers are concerned. That said, a good pain doc can mean a world of difference.

Look into cymbalta and other anti-depressants (mess with neural pathways). Look into topical agents (lidocaine patches, etc.) For you, I would particularly recommend looking long and hard at ablations and epidurals. The fact that you can't take NSAIDS and have such trouble with oral narcotics makes me think one of these would be a life saver for you. Look into Vitamin D (should be at 80). Look into a B-complex supplement. DEFINITELY look into physical therapy. When I say PT, I mean someone who is adept at small manipulations, as well--not just showing you exercises. Look into going to a neurologist for prophylactic treatment of your migraines. Since your blood pressure is a bit crazy, I would tell him that, and he might opt to go for an anti-epileptic instead of a calcium channel blocker or beta blocker. Topirimate saved me from losing my mind. If you have EDS, of course, you may need a very high dose. I take 300-400 mg. Don't let them give up on you at 100 mg (assuming you're not suffering side effects). There's also a very old migraine treatment called cyproheptadine which is an antihistamine. Given your many sensitivities, this might actually be a good treatment for you.

I also want to refer you Diana Driscoll's two studies--one suggesting diamox (an prescription anti-epileptic drug) for headaches based on the hypothesis that our headaches and sub-occipital symptoms are not migraines but symptoms of external coordinating hydrocephalous; and the other suggesting zantac (an OTC third-generation anti-histamine) and zyrtec (an OTC H2 blocker) for POTS and other symptoms. HOWEVER, I beg, beg, beg you not to treat yourself with these or any other medicines without talking with your doctor first. (By the way, that even goes for the vitamins listed in the above paragraph.) Just take the information to your doctor and ask if it's OK to do. In your situation, the zantac and zyrtec is something I actually really would ask your doctor about. The pericardial effusion, migraines, and some of the pain could be from inflammation that needs to be tamped down.

Btw, I would have thought that you were absolutely right that pain can cause a release of stress hormones which in turn are harmful for the body AND actually make pain "feel" worse. Cortisol--released in response to pain--is a natural steroid. One shouldn't be on steroids when trying to fight an infection (such as osteomyelitis). However, I need to reserve judgement until after I read a very new article, which may or may not contradict this idea: http://www.ncbi.nlm.nih.gov/pubmed/21256679. Below are some older articles you may find interesting.

http://www.ncbi.nlm.nih.gov/pubmed/2959193

http://www.ncbi.nlm.nih.gov/pubmed/21718526

http://stress.about.com/od/stresshealth/a/cortisol.htm

The point, however, is that there are a lot of non-NSAIDs and non-narcotics to get you out of pain. Please investigate those. You sound miserable, and that is not ok. Oh, and fight your insurance if they say they won't pay for Dr. Tinkle or other "out of network" specialists. Tell them that an accurate diagnosis and appropriate treatment will save them money in the long run.

Going for a walk with the doggies:)

Good luck to you.

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Mommydancer--thank you for letting me know that Dr. Tinkle does see adults. Where all does he see patients? Also, my cardiologist made my referral to Dr. Grubb in March. I am still waiting to get in to see him. Can you tell me how long you had to wait? And can you tell me what I can expect at the visit as we will be coming to Toledo from southwestern Ohio? Does he do testing while you are there or within the next day (like should we think about reserving a room somewhere when I get my appointment)? My cardiologist did not want the nurse practioner to see me--just Dr. Grubb. Did it take long to get in to see Dr. Tinkle? What can I expect from that visit? I really appreciate your information. I try to stay patient but when then when you pass out or can't seem to get things accomplished without having to rush to sit down/lie down, it raises the anxiety a bit (and I don't perceive myself to be an anxious person typically). When you have so many co-morbidities, which makes things more complex, you begin to think you're perceived as neurotic. I don't think my cardiologist even noted the fact that I was diagnosed with BHS on my health history as he never questioned me about it. I am so grateful to have found this support group. Thank you again.

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red balloon,

Thank you so much for the additional resources. I have had epidurals and RFA--none of which worked at all (I suppose because the muscles and nerves are avulsed and I have scar tissue on the SI where they once inserted). I have been though at least 6 courses of physical therapy over the course of 7 years--grueling, and specialized in pelvic floor and one that the physiatrist would only use and another the orthopedist recommended. I let them do everything/maneuver they needed to do over the past 7 years and it was only within the last few months that they discovered the muscles and nerves were avuled (my injury was 7 years ago). It was the interventional radiologist who found this with MRI and fluoroscopic films from the epidurals. So now my confidence in doctors who send you through these tortures ('assuming' a diagnosis as the root of the problem) and therapists who do these things to you (assuming the doctor has you diagnosed correctly, all the while you tough it out) is shaken.The radiologic interventionalist did put me on Lido patches and they help but only a little--but a little is better than none.

I have sought prophylactic treatment for the migraines for decades. They wouldn't try calcium channel blockers or beta blockers because my BP ran too low (not the problem now). I have a list that is 2 pages long of medications that were tried that either were not effective or had side effects that were intolerable or that I was found to be allergic to (including some antidepressants, epileptic, very high doses of Neurontin, Lyrica, Cymbalta and I could go on and on). I even tried accupuncture for both the migraines and the fibro but it didn't do a thing for me and because I bled with some treatments, the doctor backed off in the facial region. My cardiologist sent me back to neuro to R/O other neuro condition as cause of syncope but reviewing tests/exam proved negative and the neuro reviewed what had been tried and said had nothing new to offer for prophylaxis that was not tried. At one point they had me on a combo of 3 meds (I cannot recall what they were but one was Effexor) and I had some sort of reaction that caused continuous jerking of my face, head and extremeties. They had me come into the office right away and they said it was some type of reaction to the meds (some chemical in the brain--I cannot remember what they called it but would know it if I heard it). My BP was 240/120 (and that was when I ran low BP). They withdrew those medications and put me on Periactin for a week to counter the effects. I have seen 2 different pain doctors for the migraines--telling each with all earnest that I was not there for drugs--am not a med seeker. My ENT surgeon feels I have nerve damage having so many pituitary/sinus surgeries that lends to the migraines. One pain doctor put me through 3 cervical epidurals (that was the one I later found had been sanctioned by 2 states) and the other said he thought he could help by injecting something just above my brow region (and inserted a dripping cloth soaked in lido and had me sit upright in a chair for 20 minutes) and it did absolutely nothing (I left with the impression of watching/listening that they had never even done that procedure on anybody before). My husband had gone to him for back problems and he liked him (but I got the impression that he was like my former pain doctor--saw patient with insurance and saw dollar signs). My hubby had to keep going back for more and more injections--this spot/that spot--think this will get it this time--always within a short period of time. I was concerned that he was getting all these steroids when he has osteopenia that nobody checks and if the injections were successful, they should be longer lasting. Eventually, my hubby decided on his own that he wasn't doing him any good.

I don't give up, though. If my doctors know of something or somebody that is achieving good results, I am open if they believe in it/him/her. This is why I am waiting on the appointment with Dr. Grubb, the cardiologist at the University of Toledo who specializes in patients with dysautonia/POTS. My cardiologist recommended him and urged me to travel there if I could and I agreed to do whatever he recommended. I am also suspicious that the big doses of steroids that I got during the epidurals (they hit places in the lumbar and sacral spine/the SI joint and buttock trigger points) might have set me up for HTN. I developed a Cushing's appearance that still lingers a little. Whenever I go to my docs they tell me I look like somebody on steroids (moon faced)/fluid retention. As I'd mentioned I have multiple drug allergies and seem to be sensitive to stuff that I don't discover until afterwards.

I will definitely read the links you have given me and again thank you so very much for all your time.

I do take 1000 mg (plus a little that is in my calcium and magnesium tablets) of vitamin D daily after rheume found me to be vitamin D deficient to the point of having to take Rx strength vitamin D for a month.

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I'm not sure what Dr. Grubb's wait is now. I kept putting off trying to get in because I understood it to be a 6 to 8 month wait. But that made me insane so I went ahead a saw his nurse practioner, Beverly Karabin (sp?). I think I got in within six weeks. Beverly is actually a doctor, but as a PhD in addition to her nurse status. She's very knowledgable. I saw her last July and was then sent a postcard to see Dr. Grubb in December (I saw him in January). Beverly spent over an hour with me and my husband. Dr. Grubb spent over 2 hours with me in January. My visit was a consult. I'd already had a tilt table test locally. I failed miserably and the staff at my local hospital kind of "freaked out" because I don't think they ever saw anyone collapse on the first tilt. But I was in BAD shape.

In my opinion, if you're talking about a six month wait anyway, why not see Dr. Beverly Karabin first. She can order tests and arrange things with your local docs, if needed.

As for Dr. Tinkle, they got me in fairly quickly because of my neurological issues (trouble swallowing and breathing). In the same day I saw Dr. Durrani, a spine surgeon who wants to fuse my vertebrae. (that's a whole other story that you needn't worry about) He examined me but it was mostly talking and me taking notes. I think that the average wait for Dr. Tinkle is 5 months. So make an appointment and then continue to use this forum for support and information. Ask a million questions to see how others cope.

Regarding POTS... (I was diagnosed with POTS two years before my EDS diagnosis, by the way). Please stay cool and hydrated. Try some compression stockings (if your doc hasn't already given you a script for them) They really do help. I like the knee highs and sometimes wear thigh-highs on bad days. I can't tolerate the pantyhose (waist high). It's too much tightness and made my heart pound. If you have the low BP then salt, salt and more salt. My first E.P. cardiologist told me that if my food tastes like it's salty enough, put on more salt! It's tough on the esophagus, just so you know!

And just so you know, sometimes it's just a bad day and it doesn't matter what you do, my friend. Don't get discouraged. Just wait for it to pass.

Hope this helps.

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Thank you, Mommydancer.

I do stay indoors in the AC as much as I can which is hard having been one who loved the outdoors/sunshine. I will make an appointment with Dr. Tinkle and get on his list--perhaps I will still see him before Dr. Grubbs, time will tell. After reading an article cited by red balloon (Dr. Tinkle contributor) I recalled the rheume who diagnosed my BHS in 2004 spoke of my skin and its stretching and the vitiligo/texture and also informed me that activity would be a struggle with me--that I walk a fine line in doing a minute activity without causing inflammation. He told me that I could anticipate the need for joint replacements in the future and to be cautious with mobility as those with BHS have a space perception problem. Those were a few of the things he was trying to explain to me. He concurred with the rheume who had diagnosed me with fibro years before as well. But the article that I referred to mentioned that people with BHS are often mis-diagnosed with fibro. Do you know if they co-exist? I really appreciate the time and patience you have taken to respond to my questions.

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FOR THE GENERAL EDS COMMUNITY, who knows of a great EDS pain doc, osteopath, or physical therapist in the Wilmington, OH, area for RubyTuesday? She obviously needs someone who truly listens and thinks.


RubyT:
You've been through/are going through what sounds like Hell. I'm so sorry. Seems like you've been quite diligent at trying lots of things, too.

The more you speak of your symptoms, the more it sounds like EDS. Stretchy skin? Odd (soft and velvety?) texture? Goodness. Hopefully you'll get to see Dr. Tinkle soon. Write up a summary (bullet style) of your symptoms, diagnoses, and experiences with pain treatments to help them see what your situation is. Segment the summary into easy to understand sections. Keep things brief.

1000 mg/day of vitamin D puts you at what blood level of vitamin D? It's the actual blood level that matters, not the amount you take each day.

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Hi Rubytuesday1,

It's so tough when this journey first begins and doctors seem to all be contradicting one another (been there)!
Generally speaking, most doctors agree that there is no "benign" anything with hypermobility -- but a patient with EDS can show few or no symptoms. Sometimes that will persist throughout their lives. My symptoms began at the age of 46, so someone seeing me at age 20 could mistakenly call it "benign hypermobility syndrome". My son became symptomatic at age 8, though, so there is a lot of variability there. (And fibromyalgia is likely a "holding area" for conditions that are undiagnosed).

I see Dr. Clair Francomano (geneticist who specializes in EDS) -- she is in Baltimore. Love her! Her waiting time is generally about 3 months. She oversees my care (and my kids), and I have a small local group of doctors who try to handle the fallout more locally. It takes a village. ha. (BTW -- with the comment on your skin, I wonder if you may have the classic form of EDS).

The red balloon was kind enough to mention my theory (it's here: http://bit.ly/moYbIB ). Feel free to share it with your doctors. I am running clinical trials (Dr. Francomano is one of the investigators in an ongoing trial, BTW), and many important findings are coming to light. One important one for you to know -- Not only is EDS related to POTS, but we're finding that most cases of POTS are relating to mast cell disease. As the red balloon mentioned, be sure to check with your doctors on treatment. I found a couple of great groups --- here's one out of Canada: http://www.mastocytosis.ca/ Be sure to check out their Patient Experience letter (and they have a great forum). Like EDS and POTS, mast cell disease isn't all that easy to figure out either (good grief), and talking to people who have been there can be of tremendous help.

You are asking for help at an amazing time -- a time where we may actually be ABLE to help! Five years ago, we'd only be able to hold your hand and tell you that we understand. Now, we're getting some real answers.
Gentle hug,
:)

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red balloon,

Thank you again for imploring help on my behalf. I do have my levels checked about every 6 mos. and they are in the normal range with this level. When I was hospitalized overnight with one of the first near syncopal episodes (that time scared me because I'd had them 3 times before but only about once/month and I sort of blew it off to maybe needing something to eat or to fatigue or to getting too hot, as it was my first encounter with any of this), they found I was also B6 or B12 deficient (I can't recall--but I had to take that for several months but my PCP, an internist, never repeats the level when I ask--I am losing my hair like crazy--despite adding a vitamin for skin, hair and nails. I will try to create a bullet style time line of sx + txs as you suggest. That is a great idea to make things clear/and be more time efficient. I think it was Mommydancer who suggested the compression stockings and my cardiologist did Rx them for me (IV). My supplier mearsured and ordered the thigh highs and it takes both my hubby and me to get them on and off. As he travels a lot, I cannot wear them (mostly wear them to church where I've had 3 episodes of very near syncope). The downside to wearing those are: I have to wear garter belt--which shows though clothing sometimes, and it hits me right at the SI where my pain is, (and the pain I have from the hard church pews/standing/kneeling is bad enough), and once it burst a blood vessel in my foot (I don't know if it was when taking them off or putting them on) so then it even hurt to wear a shoe as the vessel was on top of the foot and it takes a while for those to calm down.
My philosophy has always been not to let my afflictions control my life but with this POTS thing--well I have to use sensibility. Before I got hurt, I loved to travel with my hubby. Now, my hubby and other family are afraid to take me anyplace because they say the trip will be too hard on me (and I know they are right). When my baby cousin died this summer, I rode with family to TN and wore my TENS, took my vicodin (and imitrex in case) and Thermacare back/neck wraps and special neck pillows but then voila--the increased fluild retention up to the knees, horrible back pain, flair of the IBS (was taking MOM, stool softeners, prune juice, minimal eating due to the distention/discomfort and using heating pad on the back and lower gut). At one point I thought I would need to go to an ER, but I didn't want focus on me when there to support my family who lost their son/nephew who was so dear. When we returned to OH, I was so miserable, I called a surgeon (fearing I had prolasped hemmorrhoids) and that's when he informed me I had big thrombosed hemmorrhoids and fistula that he suggested if didn't improve he wanted me to see a colorectal specialist at
UC. I've seen so many specialists in Cincy and Dayton area and my hubby has to take off work to take me and I get tired of so many doc appts that I've been trying to do as the docs suggest and things have improved some but all in all, the IBS is what it is.

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Dr. Dianna,
Wow! I read your article. Interstingly, when my cardio sent me to neuro (while wating on the cardio specialist in POTs appt) to R/O anything neuro, who did 'exam' and reviewed EEG and MRI/MRA of neck/brain that was done in 2008. I was not able to walk on my toes or heels independently (she had to hold my arm for support) because of imbalance. I asked her about my cognitive problems (that I'd chalked up to fibro fog--sometimes the harder I try to remember what I was saying, it is like staring at a blank piece of paper), but she dismissed it (no testing). In addition to the transphenoidal hypophysectomy and repeated insults to that region with subsequent surgeries and having cervical neuralgia (with MRI in 2003 showing some narrowing around T1 of the spine), having cervical neuralgia and the accident (car hit me with such force that it cause multiple injuries--I was pedestrian), there was a hematoma on my lower cervical/upper thoracic spine that was never more than x-rayed for fx at the time of the accident--AND I have a very small Chiari I malformation--that I asked the neuro and cardio, if in the aging process/spinal degeneration could cause any reduced blood flow to the brain/any of these sx. The cardio did not know but the neuro said 'no' it was so small. Before my second pituitary surgery in 2006, I aksed the neurosurgeon about the Chiari I malformation and he said it didn't require any intervention.

I had wondered, too, back in 2004 when that rheume who specialized in fibro saw me and added the diagnosis of BHS (when I read about BHS as he suggested, and all leads led to Ehlers-Danlos), about Ehlers-Danlos II because of the easy bruising, the episodes of vaginal bleeding to such degree that I would have to go to gyn for shots of some sort to stop the bleeding, mettromenoragia, having my son 5 weeks prematurely. My hands had once been hypermobile but in the accident the ligament was torn off my thumb and my fingers over the years have the nodes and stiffness from the osteoarthritis and both hands and feet from fluid retention. The Von Willebrands was diagnosed by a hematologist after bleeding would not stop after hours and hours of pressure dressings when 4 teeth were extracted in prep for braces. I was told I had too many teeth for my small mouth (?small maxilla). There were no positive findings but the hematologist explained because of the history of the bleeding and hemorrhages that there are 'factors' (and sub-factors?) that cannot be measured, and a later 'Von Willebrands blood test' was negative. Still I hemorrhaged after a sinus surgery again in 2004 to the extent that 2 ER doctors were not able to stop it, at one point, I lost consciousness and the ENT had to be called in from Cincy to come in, pack and balloon it overnight. When I'd had a D+C after miscarriage, I had bled so heavily that I had to have packing and again was hospitalized overnight. But when the rheume said no relation w/ my BHS + Ehlers-Danlos, I was a little confused, but none of my doctors seemed familiar with it. It was not until my sister was so concerned about the wait to get in to see Dr. Grubb that she researched POTS and came upon this link (I never had told her about my dx of BHS). I trust her as a vetrinarian, knowing that many animals (i.e. dogs) get same conditions as humans. The trouble is when questioning doctors the 'ifs', most seem reluctant and want other specialists to order the testing and make (or R/O) the diagnosis. I am planning to call Dr. Tinkle tomorrow to get an appointment and see if both rheumes can fax or send my records to him. Should I ask my cardiologist to do the same? And then I will call my PCP, tell her (if I get on the list for appt) to make a referral to Dr. Tinkle on my behalf.

The more I read, it seems the more questions I have. I truly appreciate everyone's help and support in this. Thank you, again.

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Yes, I think everyone here would agree to have your main core of doctors all share your records AND be sure that YOU keep a copy of all of your records, tests, etc.
Dr. Grubb was one of the pioneers linking EDS and POTS. If you leave Dr. Tinkle's office with a recommendation for a C1/C2 fusion, I'd recommend a trial with Diamox (methazolamide may be a good substitute if you are sensitive to sulfa -- be sure to ask your docs), and mast cell treatment. As far as I know, only 2 mast cell doctors understand the link with EDS/POTS/masto. So like many people here, our family is "winging it" with OTC's and our PCP is getting us on Gastrocrom (a mast cell stabilizer.) Mast cell stabilizers are about as inert a drug as they come! The difference in our functionality has been astonishing.
My son had gotten so thin, we were afraid he would die. And me? I was afraid I wouldn't die, I was SO miserable. Every day. What a turn-around. It looks like other doctors are understanding this link to mast cell disease now. Take a look at some mast cell forums. You'll be amazed.
I was ready to have fusion, zipperhead surgery, surgery to remove my head -- ANYTHING. But now I have no neck pain, and the POTS symptoms are easing. Mast cell treatment is for life (ugh), but at least it IS a life, if you get my drift.
Hang in, OK?
:)

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I feel bad that I have fallen so far behind in the postings. And I'm thankful to be addressed with a question. Let me point out that many of my symptoms have been dismissed before proper dx. I now have the opportunity to approach the problems and move toward possible treatments. My next rule out is Chiari Malformation. I don't know if it will help me as much as it will help my daughter. Seems that I have moved into a position where it is too late. My muscles have wasted and fatigue is overwhelming. But I am going to follow the advice of my doc and move forward hoping for some comeback. One step at a time.

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